Airway-Clearance Therapy Guidelines and Implementation

Mary K Lester RRT and Patrick A Flume MD

Introduction What Is Airway Clearance? Pulmonary Guidelines Implementing the Airway-Clearance Therapy Guidelines Implementing Airway-Clearance Therapies: A Timeline for the Newly Diagnosed Patient Implementing ACT: The Patient Who Transfers to Your Center Implementing Airway-Clearance Therapy: The Hospitalized Patient A Role for Exercise Summary

The clearance of secretions from the lungs of patients with cystic fibrosis (CF) is an important compo- nent in the fight to preserve their lung function. There is excessive inflammation in the airways of these patients, which is thought to be exaggerated by ineffective and bacterial infection. In 2005 the Cystic Fibrosis Foundation formed the Pulmonary Therapies Committee to review all of the medical literature on the various airway-clearance therapies used in treating CF lung disease. The recommendations were: an airway-clearance therapy should be performed by all patients with CF, no form of airway-clearance therapy stood out as being superior to another, and that patients may express a preference of one therapy over another. They also concluded that aerobic exercise is beneficial to patients with CF, as it is to everyone, and that exercise should be a component to the overall health routine of patients with CF. The challenge for respiratory and physical therapists together with the patient/family is to develop a plan of attack through the use of various airway-clearance therapies. The respiratory and physical therapists are integral in helping patients and families develop airway- clearance routines that aid in the removal of the secretions that cause airway obstruction. There is a wide range of airway-clearance therapies that therapists can choose from when they are teaching the patients and family members the strategies of secretion removal. The questions are: What therapy is best for what age or stage of lung disease? What therapies will the patient do? And which therapies will be covered by medical insurance? These are all fundamental questions that must be answered when guiding families in finding therapies that are effective and appropriate for each CF patient’s unique situation. Key words: cystic fibrosis, airway-clearance therapies, secretion removal, airway-clearance teach- ing, airway-clearance guidelines. [Respir Care 2009;54(6):733–750. © 2009 Daedalus Enterprises]

Ms Lester presented a version of this paper at the 43rd RESPIRATORY Mary K Lester RRT is affiliated with the Departments of Respiratory CARE Journal Conference, “Respiratory Care and Cystic Fibrosis,” held Therapy, Medicine, and Pediatrics; Patrick A Flume MD is affiliated with September 26-28, 2008, in Scottsdale, Arizona. the Departments of Medicine and Pediatrics, Medical University of South Carolina, Charleston, South Carolina. Correspondence: Mary K Lester RRT, Department of Respiratory Ther- apy, Medical University of South Carolina, 169 Ashley Avenue, PO Box The authors have disclosed no conflicts of interest. 250906, Charleston SC 29425. E-mail: [email protected].

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Introduction patient so that there is the most effective clearance of airway phlegm. Airway-clearance therapies (ACTs) have long been con- sidered an important part of care of the patient with cystic Cystic Fibrosis Pulmonary Guidelines fibrosis (CF). This is based upon our understanding of the pathophysiology of CF lung disease. There is ineffective The Cystic Fibrosis Foundation established the Pulmo- mucociliary clearance1 and, from very early in life, there is nary Therapies Committee to develop guidelines for med- obstruction of the small airways by .2 Mucociliary ications and therapies to maintain the lung health of patients clearance is an important defense mechanism of the air- with CF. Thus far, there are published recommendations ways, and patients are vulnerable to chronic infection of for medications used chronically10 and for ACTs.11 A sys- the airways. There is an exaggerated inflammatory re- tematic review of the published literature was used to de- sponse, with the airways containing bacteria, inflamma- velop these recommendations. An important realization in tory cells, and cellular breakdown products (eg, neutro- the development of the ACT guidelines was the lack of phil-derived deoxyribonucleic acid and filamentous actin large, long-term studies of airway clearance. It is not that (F-actin).3-6 All of this material increases the tenacity and there isn’t any evidence to support ACT for patients with stickiness of the airway secretions, and the airways be- CF; there are, in fact, a large number of studies, but most come further obstructed.7,8 Since the mucociliary clear- have small numbers of patients, are performed over short ance system isn’t working, it makes sense to use other periods of time (some only a single treatment), and many methods to help clear the airway secretions, if only to have no comparator. A prior systematic review concluded relieve the obstruction. In addition, these secretions con- that ACT increases mucus transport for the short-term, but tain bacteria and inflammatory mediators that continue to the authors could not draw conclusions about the long- injure the airways as well as recruit new inflammatory term effects or benefits.12 Another review noted that, de- cells. Therefore, clearance of airway secretions does more spite the “dearth of high-level evidence to support any than merely relieve obstruction; it also reduces the amount secretion clearance technique,” the lack of evidence does of infection and inflammation present in the airways. not mean lack of benefit.13 The Pulmonary Therapies Com- mittee felt that the existing evidence supporting ACT com- What Is Airway Clearance? pared to no therapies was fair in quality overall. They expressed concern that because ACT is so fundamental to ACTs are physical or mechanical means of facilitating CF treatment, a large controlled trial designed to explicitly the removal of tracheobronchial phlegm through the ex- prove the benefits of ACT could not be completed. They ternal and/or internal manipulation of air flow, and the determined that the overall benefit of ACT was moderate, evacuation of phlegm via coughing. One of the earliest based upon the various outcomes that had been studied forms of therapy, which continues to be a primary method, with a variety of therapies, including increased sputum is , or percussion and postural drain- production, increased lung function in the short-term, re- age. In 1959, British physiotherapist Jocelyn Reed first duced rate of decline of lung function, and increased ex- reported that “clapping and pressure vibrations, during long ercise tolerance. In the end, they recommended that some expirations, are the most effective forms of mechanical form of ACT be performed as a routine in all patients.11 stimulus to elimination of secretions in the treatment of There were other important findings in the systematic lung abscess, collapsed lobes, and .”9 ACTs review that led to specific recommendations by the com- have evolved over the years, using imaginative strategies mittee. First, there weren’t any data to suggest that one such as high-frequency chest-wall compression using an type of ACT was better than any other. Second, patients inflatable vest connected to an air compressor, hand-held will express preferences, and it is believed that patients are expiratory vibratory devices, and, more recently, acoustic more likely to use therapies they believe to be beneficial. waves to vibrate the mucus from the airway walls. The Finally, aerobic exercise seemed to offer benefits to a respiratory therapist has an extensive menu of airway- regimen of airway clearance in addition to the other well- clearance modalities from which to choose (Table 1), each known benefits of exercise.14 with advantages and disadvantages. The intent of this pa- per is not to discuss the techniques for delivering the nu- Implementing the merous airway-clearance modalities; however, we have Airway-Clearance Therapy Guidelines included the instructions that we give to our patients and families on these therapies (Appendix). The intent is to Now that we have recommendations regarding ACT, we help guide the therapist in choosing an ACT that is appro- must address how to implement them in our CF clinics. priate for a CF patient at different stages of life. The We must find a way to communicate these recommenda- challenge for the therapist is to match the therapy with the tions to our patients and their families, and help them find

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Table 1. Methods of Airway Clearance

Therapy Age Range Advantages Disadvantages Percussion and All ages No cost/no equipment Requires caregiver Target specific areas of lung Tiresome Does not promote independence Blowing games 18 mo to 5 y Low cost Patient must have appropriate cognitive Prepares child for future spirometry ability Appropriate coughing Ն 18 mo Teaches to cover mouth None Promotes good hand-washing High-frequency chest compression Ն 2 y Promotes independence Expensive Portable Administer nebulized medications at same time Huff Ն 3–4 y Gentle coughing technique None Active cycle breathing technique Ն 4 y Independent, inconspicuous Patient must have appropriate cognitive Can be performed anywhere, anytime ability Autogenic drainage Ն 8 y Independent, inconspicuous Patient must have appropriate cognitive Can be performed anywhere, anytime ability Handheld devices, PEP, oscillating PEP Ն 4 y Low cost Patient must have appropriate cognitive Promotes independence ability Some devices allow nebulized medications Intrapulmonary percussive ventilation Ն 5 y Can nebulize medications while performing Expensive

PEP ϭ positive expiratory pressure a method of ACT that works best for them. Given the third 2. Airway clearance is an active process. Most patients recommendation that patient preference matters, it seems will be performing their therapies on themselves in the rational to introduce the patients to all of the available out-patient setting. Even in the hospital setting, the thera- therapies. Then they can make an informed decision as to pist or parent who is performing ACT on a patient works which of the therapies they are most likely to use. How- with the patient to assist clearance of the airway secre- ever, there are important factors in the choice of therapies. tions. The patient should play an active role in making the For example, an infant is not capable of performing most therapy effective by coughing throughout and performing of the available therapies and is dependent upon others (ie, deep breath-holds and forced expiratory maneuvers at ap- parents) to perform the treatment, typically percussion and propriate times. No single method of airway clearance is postural drainage. On the other hand, a person who lives better than another. This is similar to the CF Pulmonary alone will not have a partner to assist with percussion and Therapies Committee’s second recommendation. Although postural drainage, and so will probably benefit more from no therapy has been shown better than the others, this does a type of ACT that can be performed independently (eg, not mean that one form of therapy won’t prove superior to high-frequency chest compression). Therefore, there are a the others for the individual patient. What is also apparent number of factors to consider regarding recommendations is that one form of ACT may prove useful at one time, but of specific ACT, including independence, effectiveness, another will be more effective at another time. Similarly, ease of use, flexibility, treatment durations, comfort, con- patients may be able to use one form of therapy when they venience, and interruption to daily living. It has been our are home (eg, percussion and postural drainage) but may experience that patients will benefit from more than one need a different therapy when they are away from home type of ACT, and so we introduce them to all of the living alone (eg, college). options at an appropriate time of their life (Fig. 1). Before 3. Airway clearance is boring. There is no way around discussing how to introduce various therapies to patients, this reality. ACT is tedious, monotonous, and takes time to it is useful to express our other basic tenets of ACT: perform effectively. This is another reason for patients to 1. The physician prescribes the therapy but does not have access to more than one form of therapy. necessarily know what is best. The CF team typically in- 4. Be creative. This has more than one meaning. First, cludes an expert in ACT, most often a respiratory therapist there are a number of fun activities and toys for children or a physical therapist. The therapist’s role is to work with that can serve as a method of airway clearance. Take a the patient and family to determine the most appropriate walk down an aisle of party favors and look at all of the ACT and educate them as to its proper performance. toys that behave like positive-expiratory-pressure (PEP)

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Fig. 1. Not all airway-clearance modalities are appropriate for all ages. We have a strategy of introducing airway-clearance techniques to all patients at our center. We introduce the various airway-clearance techniques based upon the patient’s age and ability to perform the therapy. This timeline approximates the appropriate age the therapy can be introduced. AD ϭ autogenic drainage. CCPT ϭ conventional chest physiotherapy. HFCC ϭ high-frequency chest compression. ACBT ϭ active cycle breathing technique. IPV ϭ intrapulmonary percussive ventilation. devices. Second, we ask our patients to perform lengthy pare them for the prolonged exhalation maneuvers they therapies several times daily. We need to work with them will need to master prior to their first spirometric test. Huff to help them figure out a way to get these therapies into cough instruction can also start during this time. their routine. Listen to the patients to learn how they spend School-age children (5 years to preteen) can begin basic their day, either at work or at school, and discuss ways to active cycle breathing techniques. Patients can be taught incorporate ACT into that schedule. We must try to adapt breath-holds and prolonged exhalation maneuvers. The huff the therapies to the patient’s needs and let the therapies be cough or forced expiratory technique can be mastered at less interruptive to their daily living. these ages, and portable devices such as PEP or oscillating PEP (eg, Acapella or Flutter) should be used. Patients need Implementing Airway-Clearance Therapies: to have access to portable devices for use while away from A Timeline for the Newly Diagnosed Patient home, such as at camp, on sleep-overs, or while being driven to school. Patients this age should be encouraged to The infant (birth to 18 months old) is completely de- participate in sports. Exercise needs to be a part of CF pendent upon the caregiver (eg, parent) for performance of patient’s daily routine. Not only does exercise improve ACT. Most of the other therapies are inappropriate for the cardiovascular health and self esteem, it can also act as a infant. Therefore, the parents of the newly diagnosed in- method of removing secretions.15 Some patients report that fant with CF should be introduced to percussion and pos- when they run, bike, or swim, these movements cause a tural drainage. The therapist will meet with the parents vibration and loosening of the mucus in the chest. There initially to teach them the technique, and will meet with have not been enough controlled trials to demonstrate that them at subsequent clinic visits to evaluate their technique exercise is an effective form of airway clearance, but the and re-educate as needed. Pulmonary Guidelines Committee found that exercise Toddlers (18 months to 4 years) are often ready to try should be considered an adjunct to a patient’s airway- high-frequency chest compression. There are currently 3 clearance routine. high-frequency chest-compression devices available on the Preteen to early teen CF patients express a need to be market: The Vest (Hill-Rom, St Paul, Minnesota), InCour- “not different” from their peers. It is common for parents age (Respirtech, St Paul, Minnesota), and SmartVest (Elec- to complain that their child “will not do anything” when tromed, New Prague, Minnesota). These devices are ex- asked about airway clearance. This is a very challenging pensive, and so we confirm with the patient’s insurance time in life because teenagers want to be independent and carrier that such durable medical equipment will be cov- to make their own decisions, and often they choose to skip ered. We introduce each of the devices to the patient and performance of ACT. But this is the most vulnerable time family at a clinic visit or in the hospital; the parent or for patients, and one in which we see a greater rate of caregiver will then be able to make an informed decision decline in their lung function.16 Patients at this age can on which machine to choose, if it is clear the patient will improve on the techniques of active cycle breathing tech- use the device. Also, at this young age the patient should nique and huff cough, and learn autogenic drainage. When be taught blowing games (eg, pinwheel, bubbles) to pre- done properly, these techniques are effective and they can

736 RESPIRATORY CARE • JUNE 2009 VOL 54 NO 6 AIRWAY-CLEARANCE THERAPY GUIDELINES AND IMPLEMENTATION be done inconspicuously. We must negotiate with the teens the patient. As the patient improves, this is now an oppor- to help them establish a routine that is effective, conve- tunity to evaluate the patient’s ability to perform airway nient, and causes little interruption in their lifestyle. They clearance on their own as well as introduce therapies they should learn that breathing techniques can be done at any have not yet tried. time and they do not require an external device or care- giver. A Role for Exercise By the time they reach adulthood (age 18 years and up), patients with CF will have been introduced to the entire A question asked frequently in the CF clinic is, “Can my ACT armamentarium, including percussion and postural 30 min of running take the place of my ACT?” Indeed, drainage, high-frequency chest compression, PEP, oscil- there are many patients with CF who participate in regular lating PEP (eg, Acapella), autogenic drainage, active cycle exercise activities that are fairly rigorous. The Pulmonary breathing technique, and intrapulmonary percussive ven- Guidelines Committee recommended aerobic exercise as tilation. All adults should have more than one technique an adjunctive therapy for airway clearance. Although there available to them, and we must work with them to estab- was not sufficient evidence to conclude that exercise was lish a routine that fits with a busy lifestyle that includes as effective as other ACT, there was evidence that exercise school, work, and family time. As their lung disease reaches does result in clearance of secretions and there is improve- a more severe stage, they must realize that they are likely ment in quality of life.15 There are additional benefits to to require increased time spent with ACT. aerobic exercise: increased endurance, increased self es- teem, and improved cardiovascular health.14 Patients with Implementing Airway-Clearance Therapy: CF who have greater fitness have better survival.17 It is our The Patient Who Transfers to Your Center perception that the patients in our clinic who exercise reg- ularly tend to have better overall health. Like many others, people with CF may move to a dif- ferent city, and will transfer their care to a different CF Summary center. When we see a new patient for the first time, we try to learn what therapies the patient is currently performing The CF Pulmonary Guidelines Committee came to the and assess their technique, providing tips for improvement same conclusion that most clinicians had already believed: as needed. We also perform a survey of their knowledge of that performance of techniques of airway clearance works all of the other therapies. Do they have knowledge of the for patients with CF. We do not have evidence that any other therapeutic options? Have they tried them? What did single method of ACT is best for all patients, but we are they like or not like about those therapies? We will then fortunate to have many therapies from which to choose. make plans for a future clinic visit to introduce them to the The opportunity for the therapist and the entire CF team is therapies they have not tried, following the same timeline to work with the patients and families to find the therapies noted above. that work for the individuals, given the myriad of resources they have and the hurdles they face. It is important to listen Implementing Airway-Clearance Therapy: to the patients as they will help us figure out what works The Hospitalized Patient best for them, and they will teach us tricks that will work for other patients as well. Patients with CF may experience acute worsening of symptoms, such as increasing cough and sputum produc- tion, often called a pulmonary exacerbation. The CF pul- REFERENCES monary guidelines on treatment of a pulmonary exacerba- 1. Boucher RC. Evidence for airway surface dehydration as the initi- tion suggest that ACTs may need to be changed during ating event in CF airway disease. J Intern Med 2007;261(1):5-16. such an event.11 In situations where the exacerbation is 2. Robinson M, Bye PT. Mucociliary clearance in cystic fibrosis. Pe- mild and out-patient therapy is planned, the patient can be diatr Pulmonol 2002;33(4):293-306. advised to increase the time spent with therapy, or to per- 3. Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild form the therapies more frequently, or even to consider an lung disease suggest ongoing infection and inflammation. Am J Re- alternative therapy. For those who are ill enough to war- spir Crit Care Med 1994;150(2):448-454. Erratum in: Am J Respir rant admission to the hospital, there is an opportunity for Crit Care Med 1995;151(1):260. therapists to become fully engaged. At the start of the 4. Birrer P, McElvaney NG, Rudeberg A, Sommer CW, Liechti-Gallati hospitalization it is most important to begin with a therapy S, Kraemer R, et al. Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med 1994; the patient finds the most comfortable. It is a time to 150(1):207-213. establish early success in clearance of secretions and to 5. Bonfield TL, Panuska JR, Konstan MW, Hilliard KA, Hilliard JB, establish a trusting relationship between the therapist and Ghnaim H, et al. Inflammatory cytokines in cystic fibrosis lungs.

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Am J Respir Crit Care Med 1995;152(6 Pt 1):2111-2118. Erratum in: monary Therapies Committee. Cystic fibrosis pulmonary guide- Am J Respir Crit Care Med 1996;154(4 Pt 1):following 1217. lines: airway clearance therapies. Respir Care 2009;54(4):522- 6. Bonfield TL, Konstan MW, Burfeind P, Panuska JR, Hilliard JB, 537. Berger M. Normal bronchial epithelial cells constitutively produce 12. van der Schans C, Prasad A, Main E. Chest physiotherapy compared the anti-inflammatory cytokine interleukin-10, which is downregu- to no chest physiotherapy for cystic fibrosis. Cochrane Database Syst lated in cystic fibrosis. Am J Respir Cell Mol Biol 1995;13(3):257- Rev 2000(2):CD001401. 261. 13. Hess DR. The evidence for secretion clearance techniques. Respir 7. Chernick WS, Barbero GJ. Composition of tracheobronchial secre- Care 2001;46(11):1276-1293. tions in cystic fibrosis of the pancreas and bronchiectasis. Pediatrics 14. Kesaniemi YK, Danforth E Jr, Jensen MD, Kopelman PG, Lefe`bvre 1959;24:739-745. P, Reeder BA. Dose-response issues concerning physical activity 8. Vasconcellos CA, Allen PG, Wohl ME, Drazen JM, Janmey PA, and health: an evidence- based symposium. Med Sci Sport Exerc Stossel TP. Reduction in viscosity of cystic fibrosis sputum in vitro 2001;33(6 Suppl):S531-S538. by gelsolin. Science 1994;263(5149):969-971. 9. Doershuk CF, editor. Cystic fibrosis in the 20th century, people, 15. Balestri E, Ambroni M, Dall’Ara S, Miano A. Efficacy of physical events, and progress. Cleveland, Ohio: AM Publishing; 2001. exercise for mucus clearance in patients with cystic fibrosis (ab- 10. Flume PA, O’Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ, stract). Pediatr Pulmonol 2004;38:316. Willey- Courand DB, et al; Cystic Fibrosis Foundation Pulmonary 16. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Reg- Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic istry, 2006 Annual Data Report to the Center Directors. Bethesda, medications for maintenance of lung health. Am J Respir Crit Care Maryland: Cystic Fibrosis Foundation; 2007. Med 2007;176(10):957-969. 17. Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF. The prognostic 11. Flume PA, Robinson KA, O’Sullivan BP, Finder JD, Vender RL, value of exercise testing in patients with cystic fibrosis. N Engl Willey-Courand DB, et al; Clinical Practice Guidelines for Pul- J Med 1992;327(25):1785-1788.

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Discussion Minnesota Protocol,1 in which they in- physiotherapy and postural drainage crease the frequency throughout the and got a lot of mucus out. If one Geller: It’s great to hear somebody treatment, and every 5 minutes they therapy is not working, try another. I talk about the practicalities of how to pop a hose and do some coughing. It have not had any patients who felt get people to do airway clearance, adds time to the therapy session, but it like doing drainage positions after vest which is neglected by a lot of people, makes the session more active, because therapy. and it’s really fundamental. You men- they’re doing true airway clearance by tioned Rule 4, airway clearance is bor- stopping to cough. I’ve had some good Flume: Or during. ing, and Rule 5, think outside the box. response with that treatment plan. I ask my patients, “Have you figured Some patients really take on the Min- Lester: Sure, or during. out a way to make airway clearance nesota Protocol, and they do their neb- fun?” As of yet, I have not had a sin- ulizer treatments at the same time. That Ratjen: The guidelines reflect our gle person give me an answer other makes vest therapy more active than lack of data on whether one airway- than, “I sit in front of the computer or passive. clearance therapy is better than an- TV,” and that’s their exercise too: us- other, and I’m a bit frustrated by that, ing the remote control. REFERENCE because we’ve been doing airway Some of my healthiest patients play clearance techniques in CF for decades wind instruments: trumpet, trombone, 1. Kempainen RR, Williams CB, Hazelwood now, and the evidence we have comes A, Rubin BK, Milla CE. Comparison of saxophone, or clarinet, but not flute: high-frequency chest wall oscillation with from rather small studies. On many instruments that create some resis- differing waveforms for airway clearance questions we’ve done large, well- tance. With wind instruments, they’re in cystic fibrosis. Chest 2007;132(4):1227- powered studies that provided good basically performing high-pressure 1232. evidence, but we haven’t done that PEP, and it’s a wonderful airway- for airway clearance. Should we just Geller: After vest therapy, how im- clearance technique. Usually a prac- give up on that, or, rather than saying, portant is the addition of postural tice session lasts about 30 minutes to “One method is probably as good as drainage? They’re usually sitting up- an hour a day, and if they like the the other, but we don’t really know,” right during vest therapy, so how im- instrument, then it’s fun. That’s one should we try to get some more evi- portant is it to add postural drainage, way to make airway clearance fun. dence? for someone who has ineffective Exercise is an adjunct to therapy, Our physiotherapists are getting to- cough? and when they get old enough, they gether to compare PEP and vest ther- can join the marching band and play apy, and to determine whether it’s go- Lester: I’ve never suggested that. and march and get exercise at the same Sometimes at the hospital we might ing to be feasible and whether you can time. That’s the only thing that I’ve add CPT [chest physiotherapy] and power the study to find an outcome found, besides putting a vest in the postural drainage to target specific measure that really shows a difference. car, that might actually be fun, at least problem areas of the lung. I still think that having stronger evi- for some patients. Unfortunately, when dence than we have now would help, they graduate high school, they usu- O’Malley: If you want to adversely and maybe some of the more sensitive ally drop the instrument and don’t play impact adherence, asking them to do techniques, such as the lung-clearance any more, and then lung function de- 2 consecutive treatments would prob- index, will help us sort this out and teriorates because they never learned ably be the way. I wouldn’t ask a pa- give us better outcome measures. The any other technique. But it was fun tient to do 30 minutes of vest therapy current evidence is as you presented while it lasted. and then spend more time doing an- it, but I still feel a little uncomfortable other therapy, at least not in home man- about this, because in many areas we Lester: On YouTube an airway- agement. say we’ll build our guidelines on the clearance video showed a duck-call- data, but lack the data for airway clear- ing contest, and the 3 finalists were Lester: We had a 5-year-old CF pa- ance. CF patients. They exhaled through a tient recently, and her main therapy vibrating duck whistle. They had fun was vest therapy. While she was an Lester: It’s an n-of-1 study some- with it and probably didn’t know that in-patient, we thought it was a good times. What works for one patient may they were doing airway clearance. Vest time to try different therapies. She was not work for another. If vest therapy therapy can be a passive therapy, and so sick and had a lot of secretions, and is the best way to do it, but the patient when I went to Minnesota last year the therapist thought vest therapy was won’t do vest therapy, then we have for adult benchmarking, they used the not being effective, so they tried chest to use other therapies.

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Flume: The guidelines committee That said, if an individual patient is 2. Airway clearance: physiology, pharmacol- did ask whether we should do a de- not improving with vest therapy and ogy, techniques, and practice. Respir Care finitive study of airway clearance. The they don’t have an effective cough, 2007;52(10):1239-1405. 3. van der Schans CP. Bronchial mucus trans- first question was to compare airway Pulmozyme’s not going to work— port. Respir Care 2007;52(9):1150-1156; clearance to no airway clearance. As nothing will work—you have to be discussion 1156-1158. much as we all know the importance able to cough it out. If they don’t have of airway clearance, the evidence to an effective cough, they might jiggle McFall:* Do you find that patients support it was only level B. The guide- the secretions around, but they won’t have a big resistance to airway clear- lines1 state that, although we might get them out. So the question is, would ance, or is it just that they do not un- want more robust data, the chance of postural drainage help? I don’t know derstand the reasons for this therapy? a study being completed successfully the answer. Or is it the time required? Patients is probably nil, because I don’t think don’t necessarily realize that they’re anybody would enroll a patient in such Volsko: We haven’t paired vest ther- needing to do their therapy. If I don’t a controlled trial. apy with postural drainage, but we take my enzymes, I notice within As for the studies of devices, the have paired postural drainage with os- 12 hours, but patients adapt very well rigor of the approval studies for a de- cillating PEP devices, such as Flutter to feeling short of breath; they get used vice is far less than that for a medi- or Acapella, and that seemed to help. to feeling congested, and they don’t cation, and that’s why device studies Mary, you brought up a really good necessarily make the connection that have few subjects. We would like more point about thinking outside the box. if they did their airway clearance, it rigorous evaluation of new devices, We use the Acapella with a mask in would be beneficial. How do you ad- but the challenge is, what would we very young patients who can’t per- dress that? like to see measured? As Bruce said, form all the postural-drainage posi- what might seem to be the most intu- tions because of reflux, and that works Lester: It’s different at different itive outcome measure (sputum vol- nicely, as it does with patients at end phases in life. With parents of a newly ume) may not be very useful. of life, who have very low expiratory diagnosed and asymptomatic infant, it flow. We get a good cough and some can be difficult to get them to buy in expectoration from those patients as and do the therapies twice a day. With REFERENCE well. 5-to-8-year-olds who are starting to 1. Flume PA, Robinson KA, O’Sullivan BP, become symptomatic and have had Finder JD, Vender RL, Wiley-Courand Rubin: If I can address this: at the their first Pseudomonas-positive cul- DB, et al. Cystic fibrosis pulmonary guide- airway clearance journal conference a ture, usually the family will buy in at lines: airway clearance therapies. Respir 1,2 that point. Teenagers don’t want to do Care 2009;54(4):522-537. year ago there was a full review about airway clearance physical ther- their therapies; they want to be incon- apy, and Cees van der Schans, who spicuous and fit in with their peers, so Newton: Would you put the patient therapies stop. When they become does the Cochrane reviews, looked at in the head-down position after vest adults and the lung disease gets more the role of postural drainage.3 It was therapy? severe, then they’re on board, and usu- clear that from the studies that were ally very adherent to therapy. At our done, which were few, there’s no ad- Geller: I’m not the right person to center we teach all the therapies by vantage with postural drainage at all, ask that question, you are. I asked that the time they’re 12 years old. Life cir- under any circumstances, other than question because before the advent of cumstances get in the way a lot of the fact that it prolonged physical ther- vest therapy we had hands-on CPT times. followed by postural drainage. With apy. There are some risks for increased intracranial pressure in small babies, vest therapy there’s no postural drain- Flume: Folks with CF are not dif- increased reflux, and discomfort in a age recommended, so the question is, ferent from the rest of us. Most of us head-down position. So in our center, did that do any good in addition to aren’t going to get on the treadmill for example, we do not use postural CPT, or did we just do it because it today, even though we know it has made sense that gravity might help? drainage. health benefits; it’s boring and it’s hard And maybe it did help in some peo- to get into that routine. ple, maybe it didn’t in others, but it was just the standard regimen: CPT REFERENCES and postural drainage. I’m not sure it 1. Airway clearance: physiology, pharmacol- would have an effect. I don’t know ogy, techniques, and practice. Respir Care * Kristin McFall, Hill-Rom, St Paul, Minne- that there’s any evidence. 2007;52(9):1070-1238. sota.

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Lester: And it’d be hard to fit in to- rather fewer devices, and certainly rel- cebo arm, but I don’t think that’s fea- day. Every day is different. I don’t atively fewer vests. Part of that might sible. think it’s a lack of knowledge of the be cost. At my institution there are In various countries throughout the clearance techniques; it’s just, “what studies underway on whether vest ther- world there’s a lot of belief in certain am I going to do at this particular apy is as effective as other techniques. techniques. In Belgium, Chevalier in- time?” That’s why if you can incor- troduced autogenic drainage and just porate sports or wind instruments into Lester: Do you emphasize physical about every patient is going to use activities of daily living, the patient exercise? that technique, and he goes from cen- will get health benefits and be doing ter to center teaching those techniques. normal activities without necessarily Davies: We emphasize physical ex- The problem I have is that there’s a realizing how much benefit they’re ercise, but not as a substitute for air- lot of belief in the field, but a lack of getting, and they’ll buy into it more. way clearance. We try to promote that data. We strongly encourage exercise as a healthy lifestyle, but certainly in and we have a long-term exercise study Marshall: I’m always struck by how the pediatric clinic we’re always say- going on in Toronto. One interesting complicated it is for pediatricians with ing, “That’s not instead of your phys- thing we see in the data from more the developmental stages and dealing iotherapy; that’s as well as.” than 6 years is a correlation between with all those little intricacies, as op- daily physical activity and lung-func- posed to internists, for whom it’s a Ratjen: In Canada the primary form tion decline in CF patients, across the little more straightforward. I’m curi- of physiotherapy is PEP, based on the whole population [unpublished data], ous about differences in what airway Toronto study of PEP versus postural so we strongly believe that exercise clearance techniques are prescribed drainage.1 It was a small study but it should be a focus. The only problem and how exercise is approached in Eu- looked rather positive, and it used a is that we have the ideal intervention rope and Canada versus the United relatively simple technique, so this is to promote that as a long-term goal, States. the primary form of physiotherapy. because exercise protocols are diffi- The cost of vest therapy is one of the cult to follow, so you have to find Davies: Something that struck me in major concerns, and there’s a lot of ways to promote regular exercise; the a couple of the talks today is that in interest in looking at that. data support that. the United Kingdom I think we use We helped our physiotherapy col- fewer devices, we certainly use vest leagues Maggie McIlwaine and Jen- therapy very much less commonly than nifer Agnew design a study to com- REFERENCES what you’re describing. In my center, pare PEP to vest therapy in a cohort of 1. Newbold ME, Tullis E, Corey M, Ross B, and at Great Ormond Street Hospital, CF patients, with pulmonary exacer- Brooks D. The Flutter device versus the where I’ve also worked, we very much bation as a primary outcome.2 I think PEP mask in the treatment of adults with more rely on the parents and the child looking at lung function is probably cystic fibrosis. Physiotherapy Canada 2005; 57(3):199-207. to administer the straightforward tech- not going to be very useful, and if I 2. McIlwaine M, Davidson G, Bjornson C, niques, as opposed to using the de- use the analogy from hypertonic sa- Smith C, Pasterkamp H, Kraemer L, et al; vices that you’ve mentioned. We have line, then I expect that pulmonary ex- University of British Columbia; Canadian them available, and we use them in acerbation is probably the outcome we Cystic Fibrosis Foundation. Long-term difficult cases, and we depend on our have to look at. It’s going to be a one- study, comparing vest therapy to positive expiratory pressure (PEP) therapy in the physiotherapy colleagues to advise us year study. The problem is that there treatment of cystic fibrosis. http://clinical on what they think is best for the child, is no placebo arm, and ideally you trials.gov/ct2/show/nct00817180. Accessed but in general I suspect that we use would like to do that study with a pla- April 24, 2009.

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